Renal - Neoplasia Flashcards
What is an angiomyolipoma?
A hamartoma
(comprised of blood vessels, smooth muscle. and adipose)
Angiomyolipomas are hamartomas associated with what condition?
Tuberous sclerosis
Angiomyolipomas are hamartomas comprised of what tissue type(s)?
Blood vessels;
smooth muscle;
adipose tissue
Angiomyolipomas are benign renal neoplasms found primarily in what organ(s)?
The kidneys
Renal cell carcinoma is a malignant epithelial tumor arising from what?
The kidney tubules
What is the classic triad of renal cell carcinoma presentation?
(1) Hematuria
(2) Palpable mass
(3) Flank pain
The classic triad of renal cell carcinoma presentation is hematuria, palpable mass, and flank pain. How often do they occur simultaneously?
Very rarely
What is the most common presenting symptom of a renal cell carcinoma?
Hematuria
Name a few paraneoplastic syndromes associated with renal cell carcinomas.
EPO secretion
Renin secretion
PTH-related-peptide secretion
ACTH secretion
True/False.
Commonly, renal cell carcinomas present as left-sided varicocele.
False.
Rarely, renal cell carcinomas present as left-sided varicocele.
Gross exam of a renal cell carcinoma reveals what?
A yellow mass
Microscopically, the most common subtype of renal cell carcinoma presents how?
Clear cytoplasm
(clear cell subtype)
Pathogenesis of renal cell carcinomas involves a loss of ______ (tumor suppressor), which leads to increased ______ (growth factor) and ______ (growth factor).
Pathogenesis of renal cell carcinomas involves a loss of VHL, which leads to increased IGF-1 and HIF-1-α.
HIF-1α increases secretion of which transcription factors?
VEGF and PDGF
Are renal cell carcinomas hereditary or sporadic?
Can be either!
Hereditary renal cell carcinomas are most commonly seen in what condition?
von Hippel-Lindau disease
von Hippel-Lindau disease typically presents in ______ (age group) and 50% present with ______ (laterality) renal cell carcinomas.
von Hippel-Lindau disease typically presents in young adults and 50% present with bilateral renal cell carcinomas.
Sporadic renal cell carcinomas classically arise in _________ (patient population) as a single tumor in the upper pole of the kidney.
Sporadic renal cell carcinomas classically arise in adult males as a single tumor in the upper pole of the kidney.
Sporadic renal cell carcinomas classically arise in adult males as a single tumor in what location?
The upper pole of the kidney
What is the major risk factor for sporadic renal cell carcinomas?
Cigarette smoke
Renal cell carcinoma TNM staging is unique in that the tumor analysis involves assessment of the tumor’s interaction with what?
The renal vein
In addition to their spread to the renal vein, renal cell carcinomas commonly spread to which lymph nodes?
The retroperitoneal lymph nodes
Wilms tumor is a malignant tumor of the ________ that is comprised of immature mesenchyme known as ________.
Wilms tumor is a malignant tumor of the kidney that is comprised of immature mesenchyme known as blastema.
True/False.
Wilms tumors are benign tumors comprised of blastema (mature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.
False.
Wilms tumors are malignant tumors comprised of blastema (immature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.
A child presents with a large, unilateral flank mass. What is the most common renal tumor in children?
Wilms tumor
Describe the clinical presentation associated with Wilms tumor.
A child with a large, unilateral flank mass, hypertension, and hematuria.
Why do children with Wilms tumor present with hypertension?
Excess renin secretion by the tumor
Most cases (90%) of Wilms tumors are ___________ (etiology).
Most cases (90%) of Wilms tumors are sporadic.
Although most cases of WIlms tumor are sporadic, Wilms tumors are also associated with some syndromes. Name three.
WAGR syndrome
Denys-Drash syndrome
Beckwith-Wiedemann syndrome
Describe the features of WAGR syndrome.
Wilms tumors
Aniridia
Genital abnormalities
Motor Retardation
WAGR syndrome is associated with deletions in what gene?
WT1
Describe the features of Denys-Drash syndrome.
(1) Wilms tumor
(2) Progressive renal disease
(3) Male pseudohermaphroditism
Denys-Drash syndrome is associated with mutations in what gene?
WT1
Describe the features of Beckwith-Wiedemann syndrome.
(1) Wilms tumor
(2) Neonatal hypoglycemia
(3) Muscular hemihypertrophy
(4) Organomegaly
Beckwith-Wiedemann syndrome is associated with mutations in what gene?
WT2 cluster
(particularly IGF-2)
What disorder is described below?
A 3-year-old child presents with aniridia, a large, left flank mass that does not cross the midline, genital abnormalities, and some lack of motor function.
WAGR syndrome
(Wilms tumors
Aniridia
Genital abnormalities
Motor Retardation)
What disorder is described below?
A 2-year-old child presents with a large, left flank mass that does not cross the midline, ambiguous external genitalia, and elevated serum BUN. Karyotyping reveals that the child is XY.
Denys-Drash syndrome
- ((1) Wilms tumor*
- (2) Progressive renal disease*
- (3) Male pseudohermaphroditism)*
What disorder is described below?
A newborn presents with umbilical hernia, hypoglycemia, overgrowth of the lower extremity musculature, and macrosomia. Two years later, the patient is found to have a large, unilateral right flank mass that does not cross the midline.
Beckwith-Wiedemann syndrome
- ((1) Wilms tumor*
- (2) Neonatal hypoglycemia*
- (3) Muscular hemihypertrophy*
- (4) Organomegaly)*
Malignancies of the lower urinary tract are defined as malignancies arising in what locations?
Renal pelvis;
ureter;
bladder;
urethra
Most malignancies of the lower urinary tract (squamous cell, urothelial, adenocarcinoma) occur in what organ?
The bladder
What is the most common type of malignancy of the lower urinary tract?
Urothelial carcinoma
(a.k.a. transitional cell carcinoma)
Urothelial carcinomas (transitional cell carcinomas) most commonly arise in what location?
The bladder
Urothelial carcinomas (transitional cell carcinomas) are most commonly seen in what patient population?
Older adults
What is the major presenting sign/symptom of urothelial (transitional cell) carcinomas?
Painless hematuria
What is the major risk factor for urothelial (transitional cell) carcinomas?
Can you name any others?
Cigarette smoke;
naphthylamine, azo dyes, long-term cyclophosphamide or phenacetin use
Urothelial (transitional cell) carcinomas are often ______focal and _______ (do/do not) recur.
Urothelial (transitional cell) carcinomas are often multifocal and do recur.
What are the two main pathways from which urothelial (transitional cell) carcinomas arise?
(1) Flat (early P53 mutations)
(2) Papillary
Which pathway of urothelial (transitional cell) carcinoma is associated with early P53 mutations?
The flat pathway
(as opposed to papillary)
Describe the progression of the flat pathway of urothelial (transitional cell) carcinomas.
Develops as a high-grade, flat tumor; then invades
Describe the progression of the papillary pathway of urothelial (transitional cell) carcinomas.
Develops as a low-grade, papillary tumor; then progresses to a high-grade tumor; then invades
Which form of urothelial (transitional cell) carcinoma begins as a high-grade tumor, invading soon after?
The flat form
Which form of urothelial (transitional cell) carcinoma begins as a low-grade tumor and then progresses to a high-grade tumor before invading?
The papillary form
Which form of urothelial (transitional cell) carcinoma is associated with early P53 mutations?
The flat form
True/False.
Urothelial (transitional cell) carcinomas are often multifocal, but they typically respond well to typical resection.
False.
Urothelial (transitional cell) carcinomas are often multifocal, and they recur.
Squamous cell carcinomas of the lower urinary tract typically involve which organ?
The bladder
What typically must occur before a squamous cell carcinoma can arise in the lower urinary tract?
Squamous metaplasia
(normal bladder and urinary tract surface is lined by urothelium)
Schistosoma haematobium is associated with what malignancy?
Squamous cell carcinoma of the bladder
Squamous cell carcinomas of the bladder associated with Schistosoma haematobium are most commonly seen in what patient population?
Egyptian males
Squamous cell carcinoma of the bladder that arises in an older woman is associated with what risk factor?
Chronic cystitis
True/False.
The major risk factor for squamous cell carcinoma of the lower urinary tract is cigarette use.
False.
The major risk factor for urothelial (transitional cell) carcinoma of the lower urinary tract is cigarette use.
Long-standing nephrolithiasis is a risk factor for what malignancy of the lower urinary tract?
Squamous cell carcinoma
___________ of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.
Adenocarcinoma of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.
A glandular malignancy arising at the dome of the bladder is most likely to be arising from what?
The urachal remnant
(The malignancy is an adenocarcinoma.)
Name an inflammatory condition that increases the risk of adenocarcinoma of the bladder.
Cystitis glandularis
Name a congenital abnormality that increases the risk of adenocarcinoma of the bladder.
Extrophy
Describe the histology of clear cell renal cell carcinomas.
Clear cells (in an acinar growth pattern) with intricate “chickenwire” vasculature
